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Arquivos de Medicina
On-line version ISSN 2183-2447
Abstract
PINTO, Pedro. Hepatic adenomas: from genotype to the clinic. Arq Med [online]. 2012, vol.26, n.2, pp.69-77. ISSN 2183-2447.
Hepatocellular adenomas (HCA) are relatively rare benign liver tumors, whose occurrence is associated with the use of oral contraceptives, being more frequent in women. In recent years there is an increasing amount of scientific evidence leading to the proposed classification of hepatic adenomas based on their genotypic and phenotypic characteristics: (1) HCA with HNF1a inactivation (somatic or germline); tumors with germline mutation are associated with MODY3 diabetes; in this context, it is frequent the appearance of adenomatosis, tumors display steatosis and the risk of malignant transformation is small; (2) AHC with activation of the gene encoding ß-catenin, most frequent in men, associated with specific risk factors (use of exogenous androgens and glycogenosis); the risk of malignant transformation is higher than in other AHC; (3) Inflammatory HCA, characterized by abundant inflammatory infiltration, dystrophic vessels, sinusoidal dilatation and ductular reaction; a subset of these tumors is associated with mutations of the gene encoding ß-catenin, with the inherent risk of neoplastic progression, (4) Unclassified AHC. In this paper we review the morphological and genetic/molecular basis of the classification presented, the characteristics of each entity, the risk of malignant transformation and the practical implications for diagnosis, prognostic evaluation and management of patients.
Keywords : Hepatocellular Adenoma; Hepatocellular Carcinoma; Genotype; Phenotype; HNF1A gene; ß-catenin.